Feb. 11, 2000 LOS ANGELES (February 09, 2000) - Ten fingers, 10 toes. This proverbial test of a normal newborn permits many parents an initial sigh of relief. But for babies born with a serious heart defect, the problem is not always as apparent as a cursory count of digits. Life-threatening conditions such as hypoplastic left heart syndrome, in which the baby is born with an underdeveloped or absent left ventricle, can present themselves with alarming speed and severity. Once, only a transplant might have saved these children, but today a series of procedures performed by cardiothoracic surgeons at Cedars-Sinai Medical Center are giving new hope to infants and their families.
“Through new surgical techniques, babies born with half a heart are buying years -- even decades -- before their hearts might fail, necessitating a transplant,” said cardiothoracic surgeon Gregory Fontana, M.D., co-director of pediatric cardiac surgery at Cedars-Sinai Medical Center. Over the last four years, more than a dozen children have successfully completed the series of operations required to repair their faulty hearts, and another 40 or 50 are at various steps of the multi-stage process. “Transplants, while sometimes the only option, present a myriad of risks and consequences. Basically, you can paint yourself into a corner.”
The surgical approach, while not new in concept, is extraordinary because medical advances in recent years have dramatically improved its outcome, according to Dr. Fontana. “In the past, the mortality rate was so high, but now kids are doing really well and leading normal lives.”
Dr. Fontana’s partner, cardiothoracic surgeon Wen Cheng, M.D., who is also co-director of pediatric cardiac surgery at Cedars-Sinai, estimates that 90 percent of babies receiving heart tranplants have hypoplastic left heart syndrome, which affects 2,000 to 3,000 babies born each year. Sometimes symptoms are picked up during pregnancy and diagnosed through a fetal ultrasound and echocardiogram. In other cases, the syndrome is detected at birth or shortly thereafter.
“What we’re offering is an effective alternative with definite advantages,” he explained. “Once you undergo a transplant, you’re faced with the implications the rest of your life. The risks include infection and rejection, and you’re faced with at least $10,000 to $15,000 a year in medical costs. No heart transplant patient has lived beyond 20 years, which is especially significant when you are considering children with their whole lives ahead of them.”
Debbie Sipos remembers the uncertainty -- and fear -- she and husband Neil faced when son Jacob was diagnosed with hypoplastic left heart syndrome nearly seven weeks after birth. Overnight, Jacob had become gravely ill. He was coughing and vomiting, and his color turned gray. His pediatrician initially suspected a viral problem, but subsequent chest X-rays confirmed the cardiac condition. Jacob was rushed to the intensive care unit.
“His left ventricle was so small, he had a hole in his septum -- creating a common valve -- and there was a 98 percent obstruction of his aorta,” she explained. “What was keeping him alive was a duct that had stayed open from the womb. His blood was pumping through a space the size of two threads.”
Debbie called her father, a physician, with the news. She and Neil were facing the same options as other parents dealing with such a dire diagnosis: letting Jacob go naturally, pursuing a heart transplant or undergoing a series of surgeries to correct the problem.
“My dad said, ‘Whatever you do, it’s better to use the baby’s own tissue,’” Debbie related. “With a transplant, it’s a long road because of lifetime dependence on immune suppressants and ongoing concern about rejection and bacterial infections. People think you get a new heart and you’re as good as new, but that’s not the case.”
The couple decided to pursue the surgical approach, and Jacob was transferred to Cedars-Sinai. “We were behind the ambulance, and we could see him. It was the hardest experience of my life,” she said.
Debbie and Neil met with Dr. Cheng -- “a remarkable man” -- to discuss the first surgery to correct the obstruction. Jacob came through the surgery with flying colors, but then couldn’t be taken off the ventilators. Then he developed unexplained high fevers and was tested for meningitis and bronchiomalacia, though no cause was determined.
“Through all this, Dr. Cheng spent hours and hours talking with us,” said Debbie. “Everyone watched over him. There was never a question they didn’t answer.”
More than a month later, Jacob went home -- three months old and 8 pounds. “It was like bringing a newborn home,” his mom remembered. Over the succeeding years, the family faced a series of challenges -- eating and sleeping problems, anxiety attacks, hairline fractures caused by depleted calcium due to medications, high fevers and bacterial infections. “He was a tough little kid, and in his own way, Jacob decided he was going to be okay.”
“There were four or five years of hell,” Debbie admitted. But those difficult memories are tempered by watching Jacob, now a happy, active almost-five-year-old. (His birthday is Feb. 11.) He underwent his second operation, a Glen, at just under one year, and his third, a Damus, to attach his aorta to the pulmonary artery, at age one and a half. His final surgery, called a Fontan, was performed June 1999.
“The final surgery was the hardest for us, because Jacob seemed in perfect health at that point -- doing better than ever,” Debbie recalled, adding. “He did great after the surgery, and now the sky is the limit.”
Tahne and Carey Lutz describe the four years preceding daughter Kennedy’s Fontan as a “roller coaster.” An echocardiogram performed shortly after birth determined a single ventricle, and the baby was transferred to Cedars-Sinai a week later for her first surgery, to attach a pulmonary artery band. The months and years that followed until Kennedy’s Fontan in July 1998 were filled with doctor visits, hospital stays, fevers and infections. Her daughter was close to death on more than one occasion, recalled Tahne, who attributes both medical care and the family’s abiding faith for seeing them through.
“It’s like a bad dream now,” she says of those difficult years. “We’re all very, very different people compared to five years ago. We’re a much closer family, and our marriage is stronger. We live each day to the fullest.”
Under Dr. Fontana’s care, Kennedy underwent a total of five surgeries and, just as with the Sipos family, the final operation was toughest. “As a parent, you think, “How can I have my healthy child risk death?”
But the results of the surgery proved phenomenal. “Kennedy’s a completely different child now -- she’s so active I have to remember I prayed for this,” she related. “She’s so happy, and I have to attribute this to the people at Cedars-Sinai. We spent a total of close to 200 days there, and the staff was so loving. When something went wrong, the nurses would cry.”
For the Weg family, there were similar highs and lows, but finally elation. Lisa Weg remembers vividly the moments after son Matthew’s Fontan, performed by Dr. Fontana in June 1999. “After the surgery, I looked at his hands, and I burst into tears. They were so pink and perfect, I couldn’t believe it. They weren’t cold anymore, and his lips were rosy red.”
It had been a long road for the family since Matthew and twin Joshua were born at 35 weeks. A fetal ultrasound at 20 weeks had detected tricuspid atresia, a condition in which the valve that opens into the right ventricle doesn’t form. Matthew was born with his left ventricle only. Within minutes, the baby was in Cedars-Sinai’s Neonatal Intensive Care Unit. He underwent pulmonary artery banding, and returned home, fairly stable, a few days later.
At six months, Matthew was doing well -- gaining weight and eating regularly -- so the Glen procedure was performed. He recovered after 10 days, and left the hospital to await his Fontan.
“When we knew Matthew had specific heart problems, we wanted to find another family to talk to that was going through the same experience,” recalled Lisa. “That didn’t happen until after the Glen, but it really helped me out. It was the first time I talked to someone who related to the eating and sleeping problems.”
Like other babies with serious heart problems, Matthew was a “finicky” eater. Lisa remembers feeling guilty when the baby ate a cookie, thinking it wasn’t nutritious enough, but at the same time relieved he ate something. It made me feel better when I talked to others about their “junk food addicts.’”
Eating problems were also a major crisis in the Melgar household, where parents Mayra and Victor would use distraction to entice food into daughter Sara. “We’d dance, sing, turn on the TV and radio to entertain and distract her,” related Mayra, adding that they syringe-fed Sara, born with no left ventricle, for 11 months. “It was so hard. She couldn’t take a bottle, because she couldn’t eat and breathe at the same time. It took more than two hours to feed her, and then she’d often vomit everything.
“The feeding problem got to be worse than the heart problem. We ended up crying most of the time.”
The Melgars eventually opted for a gastrointestinal tube to aid the feeding process, a decision they don’t regret. Sara immediately began to gain weight. “It was such a relief -- you couldn’t see her bones anymore -- and she started to walk and climb,” said Mayra.
Under Dr. Cheng’s care, Sara underwent a series of operations, ending with her Fontan in March 1999. “Now she looks great, so pink and healthy. And she’s eating everything -- pickles, onions, garlic. It’s such a milestone. We really feel hopeful now,” said Mayra of her active four-year-old, who was once listed for a heart transplant.
Perhaps Cedars-Sinai’s “smallest miracle,” Riley Rolph was born Feb. 11, 1998 at 37 weeks weighing less than three pounds. She was also born with hypoplastic left heart syndrome.
“By all the textbooks, she was not a good candidate for surgery,” explained Dr. Cheng. “Most people felt nature should take its course, and other hospitals deemed her too small for a transplant. Probably 99.9 percent of hospitals wouldn’t have tackled this case.”
Doctors Cheng and Fontana waited more than two months -- until Riley grew to 4 and a half pounds -- before performing a Norwood procedure, the first in a series of operations to correct the defect. Her Glen surgery, successfully performed in September 1999, renewed her parents’ optimism for the future. Riley did so well after her Glen, that she was able to leave the hospital in just three days -- sooner than the anticipated recovery period of five to eight days, said mom Julie Taft.
“Riley’s doing great,” said her mother, who already sees subtle differences in her daughter’s rate of growth and development since the surgery. “She has endless energy, and looks and acts normal, though she’s “peanut-size’ -- about 14 pounds at two years. Some doctors thought she’d have a lot of problems, but Riley has proved them wrong. She’s was only sick twice since her first operation.”
Now the family must await Riley’s final procedure, the Fontan, when she’s grown to about 40 pounds. Because her daughter is so tiny, Julie expects this will be when Riley is six or seven years old. Looking back and ahead, Julie expressed sentiments shared by other families as well as the physicians and staff who care for these special children.
“These kids are miracles. We’re here to support and love them.”
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